IgA Glycosylation and IgA Immune Complexes in the Pathogenesis of IgA Nephropathy

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IgA glycosylation and IgA immune complexes in the pathogenesis of IgA nephropathy.

Circulating immune complexes containing aberrantly glycosylated IgA1 play a pivotal role in the pathogenesis of IgA nephropathy (IgAN). A portion of IgA1 secreted by IgA1-producing cells in patients with IgAN is galactose-deficient and consequently recognized by anti-glycan IgG or IgA1 antibodies. Some of the resultant immune complexes in the circulation escape normal clearance mechanisms, depo...

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Pathogenesis of IgA nephropathy.

IgA nephropathy (IgAN) is initiated by glomerular deposition of polymeric IgA1(pIgA1). In IgAN pIgA production is reduced in the mucosal immune system, perhaps mediated by a mucosal gamma delta T cell defect, and mucosal IgA responses to immunisation are impaired. But pIgA1 production by the marrow is increased. Human pIgA1 has an O-glycosylated hinge region unique to circulating immunoglobulin...

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Pathogenesis of IgA nephropathy.

Immunoglobulin A (IgA) nephropathy is an immune-complex-mediated glomerulonephritis characterized by the presence of immunoglobulin A deposits in mesangial and paramesangial regions. The patients with IgA nephropathy present with varying clinical symptoms (eg, microhematuria with preserved renal function or progressive deterioration of renal functions resulting in end-stage renal disease). The ...

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IgA-containing immune complexes in the urine of IgA nephropathy patients.

BACKGROUND Sera of IgA nephropathy (IgAN) patients contain high levels of circulating immune complexes composed of IgA1 molecules with aberrantly glycosylated hinge-region O-linked oligosaccharides and IgG or IgA1 antibodies with anti-glycan or anti-hinge-region peptide specificities. Due to damaged sieving properties of the glomerular capillary wall in IgAN, these immune complexes may appear i...

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The pathogenesis and treatment of IgA nephropathy.

IgA nephropathy (IgAN) is one of the most common causes of glomerulonephritis in the world. The proliferative and crescentic forms of IgA are found in up to 30% of cases and are associated with nephritic-range proteinuria, accelerated hypertension, and accelerated decline toward ESRD. Thus, it is important to investigate the mechanism of the onset of IgAN and to identify the most appropriate tr...

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ژورنال

عنوان ژورنال: Seminars in Nephrology

سال: 2008

ISSN: 0270-9295

DOI: 10.1016/j.semnephrol.2007.10.009